Ultraschall Med 2013; 34 - PS6_10
DOI: 10.1055/s-0033-1355077

Fetal thoraco-amniotic shunting for large macrocystic congenital cystic adenomatoid malformations (CCAM) of the lung

S Schrey 1, 2, E Kelly 2, J Langer 3, A Geipel 4, H Stepan 1, GP Seaward 2, G Ryan 2
  • 1Universität Leipzig, Abteilung für Geburtshilfe, Leipzig, Germany
  • 2Mount Sinai Hospital, University of Toronto, Fetal Medicine Unit and Division of Neonatology, Toronto, Canada
  • 3Hospital of Sick Children, University of Toronto, Division of General & Thoracic Surgery, Toronto, Canada
  • 4Universität Bonn, Abteilung für Geburtshilfe und Pränatalmedizin, Bonn, Germany

Purpose: To evaluate fetal thoraco-amniotic shunting for isolated large macrocystic cystic adenomatoid malformations (CCAM) of the lung.

Methods: Retrospective study of 12 fetuses with macrocystic CCAM's, who underwent thoraco-amniotic shunting.

Results: Shunts were inserted at 24.6 (17 – 32) wks. All 12 fetuses had very large macrocystic CCAMs with marked mediastinal shift. Seven fetuses were hydropic, and 5 lesions were either very large or increasing rapidly; 4 had polyhydramnios. Shunting one cyst always decompressed the entire lesion and hydrops and/or hydramnios resolved. In one case very severe hydrops was already present at 17 wks, this fetus died 1 day after the procedure, all other 11 fetuses survived. One shunt dislodged one week after shunting, but the lesion did not re-expand. One mother ruptured membranes one week after the procedure and delivered at 33 wks by cesarean section. Two women went into labour late preterm (35+4 and 36+0 weeks), all others delivered at term. All but one of the women delivered vaginally, with no maternal complications. All babies had uneventful lobectomies shortly after birth. Pathology confirmed CCAM in all cases.

Conclusion: Fetal thoraco-amniotic shunting for large macrocystic CCAM's has an excellent outcome with a relatively low rate for complication. As fetuses with CCAM and hydrops have a very poor outcome, thoraco-amniotic shunting at a centre for fetal therapy should be offered to these parents. In cases without hydrops the benefit of shunting is discussed contradictory in the literature, but we think, that shunting should be considered, if the lesion is very large or rapidly increasing in size.